Emotional functioning of patients with neurofibromatosis tumor suppressor syndrome

Abstract

Purpose Although patients with neurofibromatosis are predisposed to multiple nerve sheath tumors that can develop anywhere in the body and cause significant morbidity (e.g., hearing loss; pain), little research has examined emotional correlates of neurofibromatosis. The purpose of this study was to examine emotional functioning among adult patients with neurofibromatosis. Methods A total of 248 patients with neurofibromatosis (neurofibromatosis 1, neurofibromatosis 2, or schwannomatosis) who received care at a specialized clinic completed validated measures to assess symptoms of depression and anxiety, level of perceived stress, and self-esteem. Results Patients with neurofibromatosis reported significantly more symptoms of depression and anxiety, higher levels of perceived stress, and lower levels of self-esteem as compared with general population norms. No significant differences were found among patients with neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis, and emotional functioning was not significantly associated with disease severity. However, increased symptoms of depression and anxiety, higher levels of perceived stress, and lower levels of self-esteem were associated with a higher frequency of self-reported medical visits in the past year (P values ≤0.05). Conclusion Neurofibromatosis appears to be associated with reduced emotional functioning. Although further research is needed, these findings suggest a role for a multidisciplinary treatment approach to address emotional distress among adult patients with neurofibromatosis.

Depression among adults with neurofibromatosis type 1: prevalence and impact on quality of life

“…The current study is the largest to date and found the highest prevalence of depression compared to prior studies, in which depression and other psychiatric disorders were reported in 21–46%. 11–13 Our data provide more compelling evidence that individuals with NF1 are at increased risk for psychiatric morbidity and suggest that this population should be screened for depression.…”

“…In a recent study of 248 adult patients with neurofibromatosis tumor suppressor syndrome (including 133 with NF1, the rest with NF2 or schwannomatosis), 37% of males and 46% of females scored at or above the cut-off of 16 on the Center for Epidemiologic Studies Depression (CESD) scale, which indicates a high likelihood of clinical depression. 11 In a previous study of 128 adult patients with NF1, one third (32.8%) had symptoms indicative of psychiatric morbidity, as assessed using the General Health Questionnaire (GHQ-12). 12 Similarly, a 12-year longitudinal study of 48 adults with NF1 found that 21% met DSM-IIIR diagnostic criteria for dysthymia and overall one-third of patients met criteria for at least one psychiatric disorder.…”

Screening and intervening: Psychological distress in neurofibromatosis type 2 (NF2)

“…Disease progression and treatment can lead to facial weakness, impaired vision, and other neurological disability. These, alongside the unpredictable nature of NF2 and concern about heritability, have a significant impact on psychological functioning often resulting in low mood anxiety social avoidance and isolation . Quality of life is reported to be similar to patients with a cancer diagnosis …”

Health-related Quality of Life of Individuals With Neurofibromatosis Type 2

“…These results parallel a prior study which showed objectively rated hearing and facial function were generally not associated with patient reported measures of emotional functioning (anxiety, depression, self-esteem, and perceived stress). (18) We also examined the effects of surgery and tumor growth on HRQoL, since these events commonly result in significant neurologic dysfunction in NF2 patients. However, we did not observe significant differences in baseline HRQoL between patients with and without prior VS surgery.…”